INTRODUCTION
The development of a lymphatic leak in the pleural space (chylous effusion or chylothorax) or in the abdominal cavity (chylous ascites) is a rare and life-threatening presentation. Although chylous effusions and ascites can occur secondary to surgical or traumatic processes, this discussion focuses on non-traumatic, congenital disorders of pulmonary or intestinal lymphatic flow that result in symptomatic lymphatic leak. Early detection of a primary lymphatic disorder improves outcome and reduces complications. Without prospective trials to guide care, we present our multidisciplinary approach to the diagnosis and treatment of congenital chylous effusions and ascites.